Sawyer Russell P, Rodriguez-Porcel Federico, Hagen Matthew, Shatz Rhonna, Espay Alberto J
Department of Neurology, University of Cincinnati, 260 Stetson Street Suite 2300, Cincinnati, OH 45219 USA.
UC Memory Disorders Center, University of Cincinnati, 234 Goodman Street, Cincinnati, OH 45219 USA.
J Clin Mov Disord. 2017 Mar 2;4:2. doi: 10.1186/s40734-017-0052-4. eCollection 2017.
Disruption of the frontal lobes and its associated networks are a common consequence of neurodegenerative disorders. Given the wide range of cognitive, behavioral and motor processes in which the frontal lobes are involved, there can be a great variety of manifestations depending on the pathology distribution. The most common are the behavioral variant of frontotemporal dementia (bvFTD) and the frontal variant of Alzheimer's disease (fvAD), which are particularly challenging to disentangle. Recognizing fvAD from bvFTD-related pathologies is a diagnostic challenge and a critical need in the management and counseling of these patients.
Here we present three pathology-proven cases of Alzheimer's disease initially misdiagnosed as bvFTD and discuss the distinctive or less overlapping historical, examination, and laboratory findings of fvAD and bvFTD, deriving analogies for mnemonic endurance from the Wizard of Oz worldview.
The Yellow Brick Road to diagnosing these disorders may be served by the metaphor of fvAD as the irritable, paranoid, and tremulous Scarecrow and bvFTD the heartless, ritualistic, and rigid Tin Man. An Oz-inspired creative license may help the clinician recognize the differential disease progression, caregiver burden, and treatment response of fvAD compared with bvFTD.
额叶及其相关神经网络的破坏是神经退行性疾病的常见后果。鉴于额叶参与了广泛的认知、行为和运动过程，根据病理分布的不同，可能会有各种各样的表现。最常见的是额颞叶痴呆的行为变异型（bvFTD）和阿尔茨海默病的额叶变异型（fvAD），这两种类型特别难以区分。从与bvFTD相关的病理中识别出fvAD是一项诊断挑战，也是这些患者管理和咨询中的迫切需求。
在此，我们展示了三例经病理证实的最初被误诊为bvFTD的阿尔茨海默病病例，并讨论了fvAD和bvFTD在病史、检查和实验室检查结果方面独特或较少重叠的表现，从《绿野仙踪》的世界观中汲取记忆要点的类比。
将fvAD比作易怒、偏执和颤抖的稻草人，将bvFTD比作无情、刻板和僵化的铁皮人，这一比喻可能有助于指引诊断这些疾病的方向。受《绿野仙踪》启发的创意方式可能有助于临床医生认识到与bvFTD相比，fvAD不同的疾病进展、照顾者负担和治疗反应。
